کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
5737769 | 1614732 | 2017 | 14 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Conditional deletion of pejvakin in adult outer hair cells causes progressive hearing loss in mice
ترجمه فارسی عنوان
حذف شرطی پژواکین در سلول های موی بیرونی بالغ باعث کاهش شنوایی متراکم در موش می شود
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کلمات کلیدی
ABROTOFOtoferlinOAEsANSDCHDIHCsOHCsDPOAEs - DPOAE هاauditory neuropathy spectrum disorder - اختلال طیفی نوروپاتی شنواییHearing loss - از دست دادن شنواییOtoacoustic emissions - انتشارات OtoacousticDistortion product otoacoustic emissions - انتشارات otoacoustic محصول تحریف شدهcalponin homology domain - دامنه هماهنگی کالپونhair cell - سلول موOuter hair cells - سلول های موی بیرونیinner hair cells - سلول های موی درونیknockout - ناکاوتAuditory neuropathy - نوروپاتی شنواییauditory brainstem response - پاسخ شنوایی مغزGasdermin - گازدرمنInner ear - گوش داخلی
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
علوم اعصاب (عمومی)
چکیده انگلیسی
Mutations in the Pejvakin (Pjvk) gene cause autosomal recessive hearing loss DFNB59 with audiological features of auditory neuropathy spectrum disorder (ANSD) or cochlear dysfunction. The precise mechanisms underlying the variable clinical phenotypes of DFNB59 remain unclear. Here, we demonstrate that mice with conditional ablation of the Pjvk gene in all sensory hair cells or only in outer hair cells (OHCs) show similar auditory phenotypes with early-onset profound hearing loss. By contrast, loss of Pjvk in adult OHCs causes a slowly progressive hearing loss associated with OHC degeneration and delayed loss of inner hair cells (IHCs), indicating a primary role for pejvakin in regulating OHC function and survival. Consistent with this model, synaptic transmission at the IHC ribbon synapse is largely unaffected in sirtaki mice that carry a C-terminal deletion mutation in Pjvk. Using the C-terminal domain of pejvakin as bait, we identified in a cochlear cDNA library ROCK2, an effector for the small GTPase Rho, and the scaffold protein IQGAP1, involved in modulating actin dynamics. Both ROCK2 and IQGAP1 associate via their coiled-coil domains with pejvakin. We conclude that pejvakin is required to sustain OHC activity and survival in a cell-autonomous manner likely involving regulation of Rho signaling.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Neuroscience - Volume 344, 6 March 2017, Pages 380-393
Journal: Neuroscience - Volume 344, 6 March 2017, Pages 380-393
نویسندگان
Suzan L. Harris, Marcin Kazmierczak, Tina PangrÅ¡iÄ, Prahar Shah, Nadiya Chuchvara, Alonso Barrantes-Freer, Tobias Moser, Martin Schwander,